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Systemic SclerosisClassification Criteria

2013 ACR/EULAR
Systemic Sclerosis Classification Criteria

The validated classification criteria for systemic sclerosis (scleroderma). Weighted scoring across skin thickening, fingertip lesions, telangiectasia, nailfold capillary abnormalities, SSc-related antibodies, pulmonary involvement, and Raynaud's phenomenon. A score of 9 or more classifies SSc. Sensitivity 91%, specificity 92%.

Original Criteria
van den Hoogen F, Khanna D, Fransen J, et al.
Arthritis Rheum / Ann Rheum Dis, 2013
Immediate Classification - Check First
If skin thickening proximal to the MCP joints is present (i.e., of the fingers AND extending proximal to the MCPs bilaterally), the patient is classified as SSc immediately without scoring. This single finding scores 9 points and alone meets the threshold. Score the remaining criteria only for patients who do not have this finding.
1
Skin Thickening of the Fingers
Select the highest applicable option. If bilateral skin thickening extends proximal to the MCPs, see the immediate classification note above (9 points).
No skin thickening of the fingers, or skin thickening not reaching the MCPs
0 pts
Puffy fingers
Diffuse swelling of fingers beyond normal joint contour, not explained by other cause. Distinct from pitting edema.
2 pts
Sclerodactyly (distal to MCPs, but not extending proximal to MCPs)
Skin thickening of the fingers distal to the MCP joints, either unilateral or bilateral. Scores 4 points - does not meet immediate classification.
4 pts
Skin thickening of fingers extending proximal to MCPs (bilateral)
This single finding classifies as SSc immediately (9 points alone = threshold). Use this option if proximal skin thickening is present. Maximum-weighted item.
9 pts
2
Fingertip Lesions
Select the highest applicable option. Pitting scars score more than digital tip ulcers.
No fingertip lesions
0 pts
Digital tip ulcers
Active or healed ulceration at the fingertip distal to the PIP joint, attributable to digital ischemia from SSc vasculopathy (not trauma or other cause). Scores 2 points.
2 pts
Fingertip pitting scars
Depressed scars at the fingertip from resolved ischemic digital tip infarcts. Scores 3 points - higher than active ulcers because scars are permanent markers of prior severe ischemia.
3 pts
3
Telangiectasia
No telangiectasia
0 pts
Telangiectasia present
Telangiectasia are macular dilated superficial blood vessels that blanch with pressure, occurring on hands, face (especially perioral and perinasal), and lips. Score 2 points if present. Distinguish from spider angiomata of liver disease.
2 pts
4
Abnormal Nailfold Capillaries
Normal nailfold capillaroscopy, or not performed
0 pts
Abnormal nailfold capillaries consistent with SSc
Nailfold capillaroscopy or dermoscopy showing SSc pattern: capillary giant loops, hemorrhages, avascular areas, or capillary loss. Score 2 points. A qualified rheumatologist or dermatologist should interpret.
2 pts
5
SSc-Related Antibodies
Anti-centromere, anti-topoisomerase I (anti-Scl-70), and anti-RNA polymerase III are the three SSc-specific antibodies that contribute to this criterion. Any one of these positive scores 3 points.
No SSc-related antibodies, or not tested
0 pts
Anti-centromere, anti-topoisomerase I (anti-Scl-70), or anti-RNA polymerase III positive
Any one or more of these three SSc-specific antibodies positive by validated assay. These are mutually exclusive in the vast majority of SSc patients (rarely more than one is positive).
3 pts
6
Pulmonary Arterial Hypertension and/or ILD
PAH diagnosed by right heart catheterization. ILD diagnosed by CT scan or chest X-ray. If both PAH and ILD are present, maximum 2 points for this domain.
No PAH, no ILD
0 pts
PAH and/or ILD present
Pulmonary arterial hypertension (by right heart catheterization: mean PAP >20 mmHg at rest with PAWP <=15 mmHg and PVR >2 WU), and/or interstitial lung disease on HRCT or chest X-ray. Either alone scores 2 points; both together still score 2 points (domain maximum).
2 pts
7
Raynaud's Phenomenon
No Raynaud's phenomenon
0 pts
Raynaud's phenomenon present
Episodic vasospasm of the fingers (and sometimes toes, ears, nose) triggered by cold or emotional stress, characterized by sequential color changes: white (ischemia), blue (cyanosis), red (reperfusion). Patient report is sufficient; physician observation not required.
3 pts
📊 Interpreting the Score
ScoreClassificationNotes
< 9Does Not Classify as SScInsufficient score. Consider early/incomplete SSc or alternative diagnosis.
>=9Classifies as Systemic SclerosisSensitivity 91%, specificity 92% in the validation dataset.

The maximum score is 19 (9 + 3 + 2 + 2 + 3 + 2 + 3 = 24 if all items present simultaneously, but skin thickening proximal to MCPs alone reaches threshold). These criteria do not apply to patients with SSc-like disorders not explained by SSc (e.g., nephrogenic systemic fibrosis, scleroderma-like GVHD, diabetic cheiroarthropathy, eosinophilic fasciitis).

💡 Pearls and Pitfalls
✓
Skin thickening proximal to MCPs alone classifies SSc. This single finding scores 9 points - equal to the entire threshold. Diffuse cutaneous SSc patients with truncal involvement always meet criteria immediately. The scoring system is most useful for limited cutaneous SSc (lcSSc) where proximal extension is absent.
✓
Raynaud's phenomenon (3 pts) plus SSc antibody (3 pts) = 6 points. A patient with Raynaud's and positive anti-centromere is already at 6 of the 9-point threshold. Adding sclerodactyly (4 pts) or telangiectasia (2 pts) reaches or exceeds the threshold - this is a common early lcSSc presentation.
⚠
Exclude SSc mimics before applying these criteria. Eosinophilic fasciitis, diabetic cheiroarthropathy, nephrogenic systemic fibrosis, scleredema, and scleromyxedema can produce skin thickening that mimics SSc. These conditions are explicit exclusions in the criteria development dataset.
🔮 Evidence

The 2013 ACR/EULAR criteria were developed by van den Hoogen, Khanna, Fransen, Johnson, and colleagues, published simultaneously in Arthritis and Rheumatism and Annals of the Rheumatic Diseases in 2013. They replaced the 1980 ACR criteria that had high specificity but poor sensitivity for early and limited SSc. The new criteria were validated in 1656 SSc cases and 1883 controls, achieving sensitivity 91% and specificity 92%.

View References▾
1
van den Hoogen F, Khanna D, Fransen J, et al. 2013 classification criteria for systemic sclerosis: an American College of Rheumatology/European League Against Rheumatism collaborative initiative. Arthritis Rheum. 2013;65(11):2737-2747. Original criteria development and validation.
For classification purposes only. Score of 9 or more classifies systemic sclerosis. Excludes skin thickening not explained by SSc. Not diagnostic - requires clinical context and exclusion of SSc mimics.
🔗 Related
🤚
mRSS
Modified Rodnan Skin Score - monitor skin disease
→
SSc Classification Score
0
/ 19+
036915+
Score items above (threshold: 9+)
Skin thickening of fingers
0 pts
Fingertip lesions
0 pts
Telangiectasia
0 pts
Nailfold capillaries
0 pts
SSc antibodies
0 pts
PAH / ILD
0 pts
Raynaud's phenomenon
0 pts
Clinical Interpretation
Score all seven domains. If skin thickening extends proximal to MCPs bilaterally, this alone scores 9 points and classifies as SSc immediately.
Reviewed by Mahiar Rabie, MS, MD · AutoimmuneCalc